Splenectomy for breast carcinoma diffusely metastatic to the spleen presenting as severe transfusion-dependent anaemia and thrombocytopaenia.

We report a 48-year-old woman with metastatic infiltrating lobular carcinoma of the breast. Though her metastatic disease remained stable, she was repeatedly admitted for symptomatic anaemia and treated by red blood cell and platelet transfusions with increasing frequency as time elapsed. Abdominal examination and ultrasound revealed splenomegaly (27 cm span). A bone marrow biopsy showed fibrosis and foci of metastatic carcinoma. Splenectomy ameliorated her transfusion-dependent anaemia and thrombocytopaenia. Histopathology revealed multiple foci of metastatic carcinoma and scattered foci of extramedullary haematopoiesis. Differential diagnosis of anaemia and thrombocytopaenia in patients with cancer include bone morrow involvement by cancer cells, iron-deficiency anaemia, microangiopathies and chemotherapy suppression of haematopoiesis. Splenic involvement with cancer is common in patients with multivisceral disease. Many may regard transfusion-dependent severe anaemia and thrombocytopaenia as an end-stage disease in these patients. Nevertheless, palliative splenectomy should be considered in patients with possible hypersplenism who will otherwise survive for a relatively prolonged period of time.

The prognostic significance of heparanase expression in metastatic melanoma.

BACKGROUND: Heparanase expression is induced in many types of cancers, including melanoma, and promotes tumor growth, angiogenesis and metastasis. However, there is insufficient data regarding heparanase expression in the metastatic lesions that are the prime target for anti-cancer therapeutics. To that end, we examined heparanase expression in metastatic melanoma and its correlation with clinical parameters. RESULTS: Heparanase staining was detected in 88% of the samples, and was strong in 46%. For the entire cohort of metastatic melanoma patients, no apparent correlation was found between heparanase staining intensity and survival. However, in a sub group of 46 patients diagnosed as stage IVc melanoma, strong heparanase staining was associated with reduced survival rates [hazard ratio=2.1; 95%CI 1.1-4.1, p=0.025]. MATERIAL AND METHODS: Paraffin sections from 69 metastatic melanomas were subjected to immunohistochemical analysis, applying anti-heparanase antibody. The clinical and pathological data, together with heparanase staining intensity, were evaluated in a logistic regression model for site of metastasis and survival. Slides were also stained for the heparanase-homolog, heparanase-2 (Hpa2). CONCLUSIONS: Heparanase is highly expressed in metastatic melanoma and predicts poor survival of stage IVc melanoma patients, justifying the development and implementation of heparanase inhibitors as anti-cancer therapeutics.

Expression of heparanase in soft tissue sarcomas of adults.

BACKGROUND: Heparanase is an endo-ß-D-glucuronidase that cleaves heparan sulfate chains of proteoglycans, resulting in the disassembly of the extracellular matrix. Heparanase has a central role in the development of various tumors, and its expression has been associated with increased tumor growth, angiogenesis and metastasis, but there is insufficient information about the function of heparanase in sarcomas. STUDY AIMS: 1) To evaluate heparanase levels in adult soft tissue sarcomas (STS); 2) To examine the correlation between heparanase levels and pathological and clinical parameters and treatment outcome. METHODS: Pathological specimens of primary or metastatic STS were subjected to immunohistochemical analysis applying an anti-heparanase antibody. The clinical and the pathological data, together with the data of heparanase levels, were evaluated in a logistic regression model for tumor recurrence and survival. RESULTS: One hundred and one samples were examined, 55 from primary tumors and 46 from metastatic sites. A high expression of heparanase was observed in 29 (52.7%) and 22 specimens (47.8%), respectively. There was no statistically significant difference between heparanase expressions in the primary vs. metastatic sites of tumors. Moreover, no correlation was observed between heparanase staining and tumor aggressiveness, tumor recurrence or patient survival in various groups of patients. CONCLUSION: Expression of heparanase was observed in 50% of the STS, in various histological subtypes. A larger study with homogenous groups of specific sub-types of STS or stages of disease is required to validate over-expression of heparanase as a marker of disease aggressiveness.

Association between esophageal leiomyomatosis and p53 mutation.

Li-Fraumeni syndrome is a cancer predisposition syndrome associated with a variety of neoplasms, mainly soft tissue sarcoma, premenopausal breast cancer, brain tumors, adrenocortical carcinoma, and leukemia. Esophageal leiomyomatosis involves the presence of several rare benign neoplastic lesions composed of proliferating smooth muscle cells in the esophageal wall. The current case report presents a patient with recurrent diffuse leiomyomas of the esophagus and confirmed p53 mutation with clinical criteria of Li-Fraumenilike syndrome.